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Lecture 20

Tumors of the Nervous System

QuestionAnswer
(T or F) In children, CNS tumors are the third most common maliganancy. False. CNS tumors are the second most common malignancy in children.
Where are majority of CNS tumors located in the pediatric population? 60-70% are located in the infratentorial regions such as the cerebellum and brainstem.
Where are majority of CNS tumors found in the adult population? 70% are located in the supratentorial region (cerebral hemispheres).
What types of CNS tumors are Neurofibromatosis type 1 patients at increased risk for? (1) Neurofibroma (2) Optic nerve glioma (3) Meningioma (4) Malignant peripheral nerve sheath tumor (MPNST)
What types of CNS tumors are Neurofibromatosis type 2 patients at increased risk for? (1) Bilateral vestibular schwannoma (2) Peripheral schwannoma (3) Meningioma (4) Ependymoma
What type of CNS tumor are Tuberous Sclerosis patients at increased risk for? Subependymal Giant Cell Astrocytoma (SEGA)
What type of CNS tumor are Von Hippel-Lindau patients at increased risk for? Hemangioblastoma
What type of CNS tumor are Li-Fraumeni syndrome patients at increased risk for? Gliomas
What type of CNS tumor are Gorlin patients at increased risk for? Medulloblastoma
What types of CNS tumors are Turcot patients at increased risk for? (1) Medulloblastoma (2) Glioblastoma
What type of CNS tumor are Cowden disease patients at increased risk for? Dysplastic gangliocytoma of the cerebellum
What type of CNS tumor are Rhabdoid tumor predisposition syndrome patients at increased risk for? Atypical Teratoid/Rhabdoid Tumor
What type of CNS tumor are MEN type 1 patients at increased risk for? Pituitary adenoma
What types of CNS tumors are associated with ionizing radiation exposure? (1) Meningiomas (2) Gliomas (3) Maignant nerve sheath tumors
(T or F) Meningiomas have a higher incidence in men than women. False. Meningiomas are more common in women than men (2:1)
(T or F) Gliomas have a higher incidence in men than women. True. Gliomas are more common in men than women (3:2).
Virus associated with Primary CNS lyphoma in immunocompromised patients EBV
Most common clinical presentation of CNS tumors (1) Epilepsy (2) Focal neurological signs (3) Signs of elevated ICP (Headache, vomiting, confusion, papilledema) (4) symptoms of hydrocephalus (Headache, nausea, vomiting, decreased conscioussness, abnormal enlargement of head in infants).
Symptoms and signs of increased ICP (1) Headache (2) Vomiting (3) Confusion, decreased consciousness, and coma (3) Papilledema
(T or F) Majority of CNS grow by expansion with defined tumor-brain borders. False. Majority of CNS tumors grow by infiltrative growth with individual tumor cell invasion of brain structures.
CNS tumor associated with a "butterfly lesion" Glioblastomas
Modes of tumor spread (1) Along white matter tracts (2) Along pial membrane (3) Along perivascular space (Virchow-Robin) (4) Across ependyma and ventricular lining
(T or F) Extraneural metastases of CNS are extremely rare. True. Malignant tumors have been reported to metastasize after operative procedures.
The most common CNS tumor in all age groups Gliomas represent approximately 45% of all CNS tumors.
WHO grade for a glioblastoma WHO grade IV. It is the most malignant glioma in adults with a poor prognosis. Aver survigial is 12-15 months.
Common location of pilocytic astrocytomas Midline sturctures such as the cerebellum, optic nerve and chiasm, 3rd ventricle region/hypothalamus, and brainstem
Peak age of onset of pilocytic astrocytomas 10-12 years. One of the most common tumors in children.
Type of pilocytic astrocytoma characteristic of Nuerofibromatosis type 1 (NF1) Optic nerve glioma
Treatment of pilocytic astrocytomas Typically can be treated with surgical resection; however, tumors located in the 3rd ventricle/hypothalamus region may benefit from radiation and chemotherapy.
Four histopathological features used for grading malignancy of diffuse astrocytomas AMEN criteria: A (Atypia), M (Mitoses), E (Endothelial proliferation), N (necrosis)
Based on the AMEN criteria, what are the positive malignant features of Astrocytomas? Atypia
(T or F) Diffuse infiltrating astrocytomas are more common in adults than children. True. Typically present during the 5th to 7th decade of life.
Based on the AMEN criteria, what are the positive malignant features of Anaplastic Astrocytoma? (1) Atypia (2) Mitotic activity
Based on the AMEN criteria, what are the positive malignant features of Glioblastoma? (1) Atypia (2) Mitotic activity (3) Necrosis and/or (4) Endothelial proliferation
Define secondary glioblastoma A tumor progressing from a lower grade astrocytoma (grade II or III)
Age of diagnosis of Primary Glioblastoma versus Secondary Glioblastoma Primary Glioblastoma: 55-60 years and Secondary Glioblastoma: 40 years
(T or F) Majority of glioblastoma cases are primary glioblastomas. True. 95% of cases are primary glioblastomas.
Typical location of Oligodendrogliomas Supratentorial areas, most commonly frontal and temporal lobe
Clinical presentation of Oligodendrogliomas The great majority of oligodendrogliomas have a slow clinical evolution and is associated with a long history of seizures.
Peak age of incidence of Oligodendrogliomas 4th and 5th decade
Type of CNS tumor commonly found in the frontal and temporal lobe and associated with a long history of seizures. Characteristic neuroimaging features include frequent micro and macrocalcifications. Oligodendrogliomas
(T or F) Oligodendrogliomas without chromosomal deletion of 1p and 19q have a better prognosis than Oligodendrogliomas with 1p/19q chromosomal deletions. False. Oligodendrogliomas with 1p/19q chromosomal deletions respond better to chemotherapy and longer disease-free time after chemotherapy. Patients survive longer.
Genetic abnormality associated with Oligodendrogliomas The most common genetic alteration associated with Oligodendrogliomas are 1p/19q chromsomal deletions.
Common histologic features of CNS embryonal tumors (1) High cellularity (2) Primitive-appearing small cells (3) High mitotic activity
Duration of symptoms to diagnosis of Primary Glioblastoma versus Secondary Glioblastoma Primary Glioblastoma has a short clinical presentation with an average of 1.7 months. Secondary Glioblastoma progresses from a lower grade astrocytoma with an average duration of symptoms to diagnosis of greater than 25 months.
WHO grade for CNS embryonal tumors WHO grade IV
Types of CNS embryonal tumors (1) Medulloblastomas (2) CNS Primitive Neuroectodermal Tumors (Medulloepithelioma and Ependymoblastoma) (3) Atypical Teratoid/Rhabdoid Tumor
(T or F) CNS embryonal tumors have a high tendency for cerebrospinal fluid seeding. True
Most common CNS embyronal tumor Medulloblastoma
Most common location of Medulloblastomas Cerebellum
Genetic alterations associated with Medulloblastomas (1) Isochromosome 17q (2) Loss of chromosome 17q in a different locus than TP53 (3) Amplification of c-myc
CNS tumor arising from meningothelial cells. May occur sporadically or in asociation with Neurofibromatosis type 2. Meningiomas
(T or F) There is an high association with Meningiomas with Neurofibromastosis type 2 in childhood. True. Approximately 40% of children with meningiomas have Neurofibromatosis type 2.
Gender predilection of Meningiomas in adults Marked female predominance (3:1)
Common location of Meningiomas (1) Convexity of cerebral hemispheres (2) Parasagittal regions (2) Skull base (3) Sphenoid wing (4) Spine
Common symptoms of Meningiomas Seizures and focal deficits are common symtpoms of Meningiomas. These tumors are extra-axial tumors and tend to compress the underlying brain/spine parechyma.
Meningioma variants with WHO grade III classification These tumors are classified as malignant: Rhabdoid, Papillary, and Anaplastic
Meningioma variants with WHO grade II classification WHO grade II tumors tend to recur. (1) Chordoid (2) Clear cell (3) Atypical
Extranodal non-Hodgkin's lymphoma arising in the CNS in the absence of obvious lymphoma outside the nervous system. Primary CNS lymphomas
Common location of Primary CNS Lymphomas (1) Supratentorial areas (deep white matter) (2) Periventricular regions (3) Basal ganglia (4) Posterior fossa
(T or F) Primary CNS Lymphomas are unlike most primary CNS tumors because the lesions are often multiple and multifocal, simulating metastatic tumor. True. Multiple lesions are particulary prevalent in the AIDS and post-transplantation population.
Most common subtype of Primary CNS Lymphomas Large B-cell lymphomas
Virus associated wtih Primary CNS Lymphomas in immunocompromised patients EBV
Subtype of pituitary adenoma characterized by amenorrhea and galactorrhea Prolactin-secreting adenomas (Prolactinomas)
Subtype of pituitary adenoma characterized by acromegaly and/or gigantism GH-Secreting adenoma (Somatotrophinomas)
Subtype of pituitary adenoma manifesting as Cushing's disease ACTH-secreting adenomas (Corticotrophinomas)
Clnically non-functional adenomas (1) Gonadotrophinomas (2) Silent adenomas (3) Null-cell adenomas
Signs and symptoms of Clnically non-functional adenomas Most Clnically non-functional adenomas present with signs and symtpoms of a tumor mass growing and compressing the sellar structures: headaches, visual loss, hypopituitarysm.
5-year survival of Pituitary adenomas 78.1%
Most common intracranial tumor Metastatic tumors of the CNS
Most common location of Metastatic tumors of the CNS 80% of brain metastases are located int he arterial border zones of cerebral hemispheres (cortical-white matter junction), 15% in cerebellum, and 3% in basal ganglia
Most common primary tumor site of intracranial CNS metastases Lung (50%)
Most common primary tumor sites of intracranial CNS metastases (1) Lung-50% (2) Breast-15% (3) Skin/Melanoma-11% (4) Unknown primary site-11%
Most common primary tumor site of intraspinal CNS metastases Breast (22%)
Most common primary tumor sites of intraspinal CNS metastases (1) Breast-22% (2) Lung-15% (3) Prostate-10% (4) Malignant lymphomas/leukemias-10%
Created by: UVAPATH2